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Background and Aims Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematopoietic stem cell disorder characterized by the presence of abnormal red blood cells and an increased risk of hemolysis.

Methods The case involved an 83-year-old man with a left humeral neck fracture who had been diagnosed and treated for PNH for 10 years. The patient was a high-risk patient with a history of both hemolytic and thrombotic symptoms, which were suppressed by treatment with the monoclonal antibody eculizumab. Surgery was performed with an intramedullary nail through the proximal end of the humerus. Given the exacerbation of PNH, light sedation with midazolam and a superior nerve trunk block with 5 mL of 0.5% levobupivacaine was performed. No significant exacerbation of PNH symptoms or hematoma formation was observed. He was discharged from the hospital on postoperative day 2.

Results There are no reports of surgical experience with peripheral nerve blocks in patients with PNH. Anesthetics or high-dose opioids for surgical management should be a risk factor for an episode of hemoglobinuria by sleep induction, as nocturnal exacerbation of hemoglobinuria has been attributed to carbon dioxide retention and blood acidosis leading to complement activation. Because the superior nerve trunk block is a superficial technique among brachial plexus blocks, the risk of hematoma formation was considered low. Treatment with monoclonal antibody could have facilitated the management of the disease and avoided perioperative problems.

Conclusions We experienced a case of PNH patient who underwent humeral head fracture surgery under regional anesthesia and light sedation.

Attachment ESRA2023_Casereport_IC.pdf